Postgrad MedJ3 1995; 71: 480-504 C) The Fellowship of Postgraduate Medicine, 1995 Short reports Postgrad Med J: first published as 10.1136/pgmj.71.838.480 on 1 August 1995. Downloaded from Angiography and the aetiology of failure

Carl IO Brookes, Paul Hart, Bruce E Keogh, John GF Cleland

Summary substances and there was no family history of The diagnosis of idiopathic dilated car- ischaemic heart disease. He was known to have diomyopathy should not be made without retinitis pigmentosa (as was his sister) but he first performing a coronary angiogram. was not ataxic or deaf. He had no other relevant If the cause of is unknown past medical history. At the time of presenta- this should be stated rather than attri- tion he was taking captopril 12.5 mg bid and buting the cause to dilated cardiomyo- frusemide 80 mg od. pathy. Severe ventricular dysfunction On examination he was short of breath on may improve dramatically after revas- minimal exertion with a sinus of cularisation in some cases of coronary 100 beats/min and a blood pressure of 100/ disease. Preservation of R waves on the 60 mmHg. The venous pressure was elevated surface electrocardiogram suggests the to the ear lobes with pulsatile hepatomegaly, presence of hibernating myocardium but ascites, and pitting oedema to the thighs. thallium scintigraphy or positron emis- Auscultation revealed a third heart sound but sion tomography scanning should be no regurgitant murmurs. Fundoscopy con- employed to investigate this further. firmed retinitis pigmentosa but the rest of the neurological examination was unremarkable. Keywords: coronary angiogram, heart failure Initial investigations revealed a normal full blood count without an eosinophilia. The urea and electrolytes, thyroid function tests, plasma Recent studies have highlighted the inaccuracy glucose, erythrocyte sedimentation rate and ofmaking a diagnosis ofheart failure by clinical C-reactive protein were normal. His total means alone."2 The aetiology of heart failure cholesterol was 2.6 mmol/l with a normal also has important therapeutic and prognostic profile. Radiologically he had implications2'3 and cannot be ascertained with- (CTR 19/29) with upper lobe venous disten- out the help of additional investigations.4 The sion. Electrocardiogram (ECG) showed sinus striking impact of angiotensin-converting rhythm, with no pathological Q waves and no enzyme (ACE) inhibitors on those patients evidence of left . with left ventricular systolic dysfunction and demonstrated biventricular http://pmj.bmj.com/ the potential benefit of surgery in others5'6 dilatation with global dysfunction (LVEDD reinforce the need to pursue clarification of the 62 mm) and mild mitral regurgitation (figure underlying diagnosis. 1). We present the case of a young man with a The normal inflammatory indices made an globally hypokinetic left which dem- active unlikely and there were no onstrates some of the pitfalls of relying on features to suggest ischaemic heart disease. A

clinical evaluation alone. associated with retinitis pig- on September 30, 2021 by guest. Protected copyright. mentosa, as occurs in the Kearns- Sayre syn- Case report drome,7 was possible but the lack of other neurological abnormalities virtually excluded A 48-year-old man, born in Pakistan but resi- this diagnosis. A diagnosis ofidiopathic dilated dent in the UK for over 20 years, presented cardiomyopathy seemed likely and he was with symptoms and signs of severe heart treated with an increase in his medical therapy. Department of failure. He had recently been discharged from A serology screen for toxoplasmosis, cox- , his local hospital with a diagnosis of dilated sackie B and Borrelia was negative. Auto- Hammersmith cardiomyopathy based on clinical and echocar- antibodies including anti-cardiolipin antibody, Hospital, Du Cane diographic findings. serum ferritin, serum vitamin A, serum Road, London, W12 His current illness began four months prior phytanic acid levels and urine screening for OHS, UK to presentation with episodic shortness of homocystinuria were also normal. He exercised CIO Brookes Bruce P Hart breath and sputum production. He received for four minutes of a modified protocol BE Keogh multiple courses of antibiotics with no effect. stopping because of shortness ofbreath with no JGF Cleland Following this he travelled to India where he chest pain or significant ECG changes. became increasingly short of breath with Coronary angiography revealed severe triple Correspondence to associated ankle swelling. He was treated by a vessel disease with global left ventricular JGF Cleland, Clinical The left ventricular Research Initiative In Heart local physician with a series of intramuscular hypokinesis (figure 2). Failure, University of injections of unknown composition but he end-diastolic pressure was 25 mmHg and car- Glasgow, Glasgow G2 8QQ, continued to get worse. Direct questioning diac index 1.4 I/min/m2. The ejection fraction UK revealed no history of chest pain or palpita- on radionuclide ventriculography was 16"\. Accepted 15 February 1995 tions. He did not smoke, abuse alcohol or illicit Positron emission tomography (PET) sug- Angiography and the aetiology of heart failure 481 Postgrad Med J: first published as 10.1136/pgmj.71.838.480 on 1 August 1995. Downloaded from

A-..

Figure 1 End-diastolic B-mode and M-mode echo- cardiographic frame demonstrating a dilated poorly contracting left ventricle.

gested widespread areas of non-contractile but viable myocardium. Despite a dramatic diuresis with metolazone and frusemide and some symptomatic benefit on lisinopril 20 mg od his improvement was not sustained. On the basis of his PET scan he was referred for coronary artery bypass surgery rather than cardiac transplantation. Although his postoperative course was stormy he was discharged home on the 17th postoperative day. Six months later his ejection fraction improved to 260% and at one year he is in NYHA class 1, with an ejection fraction of 42%, requiring no diuretic and being main- tained on lisinopril alone. Discussion This report indicates the dangers of relying on clinical features alone to make the diagnosis of . Figure 2 Coronary angiography showing an occluded The aetiology of dilated cardiomyopathy is right coronary artery (arrow 1), an occluded left anterior descending artery (arrow 2) and a tight proximal poorly understood,8 although recent work sug- stenosis of the circumflex artery (arrow 3). gests that 20% ofcases may have a familial form http://pmj.bmj.com/ ofthis disease which can partly be explained by a deletion in the promoter sequence ofthe gene that codes for cardiac dystrophin.9 Benton's study'0 30.30° of patients with a However, the clinical diagnosis of dilated discharge diagnosis of dilated cardiomyopathy cardiomyopathy depends on the exclusion of had significant as other causes of ventricular dysfunction includ- defined by more than 500° stenosis in one or

ing specific myocardial diseases, valvular heart more main coronary arteries. on September 30, 2021 by guest. Protected copyright. disease and important coronary artery disease. These studies indicate that potentially Population-based studies show that the treatable coronary artery disease will be missed majority of patients with a presumptive diag- in a large proportion of patients with a nosis of dilated cardiomyopathy never undergo presumptive diagnosis of dilated cardiomyo- coronary angiography, making the potential for pathy if coronary angiography is not per- overestimating the prevalence of this condition formed. However, coronary artery disease and large. In a review by Benton et al'0 the authors dilated cardiomyopathy are not necessarily highlight eight population-based studies of mutually exclusive and it remains a matter of dilated cardiomyopathy, in five of which the individual judgement as to whether the severity frequency of angiography was less than 500% of coronary artery disease is sufficient to (range 34-480%), the frequency ofangiography account for the degree of left ventricular dys- not being stated in the other three. Given that function in these cases. both black race and low socioeconomic status The case above also highlights the potential are associated with a lower likelihood of angio- of new imaging modalities to differentiate graphy, this may explain, in part, the supposed hibernating from infarcted myocardium. increase of dilated cardiomyopathy in these Although coronary angiography is the most groups. 1-13 accurate investigation for the detection of Furthermore, Figulla et al'4 found that only obstructive coronary artery lesions, it cannot 6600 ofpatients referred for angiography with a distinguish between viable and non-viable presumptive diagnosis of dilated cardiomyo- myocardium. Similarly, while well preserved R pathy on the basis of non-invasive investiga- waves on the surface ECG do increase the tions were correctly diagnosed. Similarly, in likelihood of finding viable myocardium, the 482 Brookes, Hart, Keogh, Cleland

presence of Q waves do not indicate an absence ofviable myocardium. 15 A variety oftechniques Learning point are being investigated which have some value in predicting areas of asynergy that will improve Potentially treatable coronary artery disease may Postgrad Med J: first published as 10.1136/pgmj.71.838.480 on 1 August 1995. Downloaded from be missed in patients with a presumptive after revascularisation. 16-18 Initial studies sug- diagnosis ofdilated cardiomyopathy ifcoronary gest that thallium scintigraphy and PET scan- angiography is not performed ning may be equally sensitive in the detection of hibernating myocardium but PET may be more specific.'9'20 However, it must be emphasized that it is not yet clear whether improvements in regional ventricular contrac- tion translate into benefits in terms of mor- Conclusion bidity and mortality. The economic repercussions of identifying A confident diagnosis of dilated cardio- and treating hibernating myocardium need to myopathy should not be made without cor- be addressed. Firstly, it needs to be proven that onary angiography. The lack of Q waves on the complex imaging modalities are more reliable surface ECG in patients with severe coronary than, for example, the surface ECG in predict- artery disease and poor ventricular function ing benefit from surgery. Secondly, several may indicate the existence ofunderlying hiber- small uncontrolled studies21-23 have indicated nating myocardium. If this is supported by that revascularisation in patients with poor left either PET or thallium imaging, coronary ventricular function and severe coronary artery artery bypass surgery should be considered. disease may improve prognosis which if The benefits of revascularisation may only confirmed, will lead to increasing surgical become apparent after weeks or months and the demand and longer waiting lists for bypass mortality in the immediate postoperative procedures. The plus side, however, is a poten- period is likely to be high, especially in tial reduction in the number of patients refer- elderly patients with widespread atherosc- red for cardiac transplantation. lerotic disease.

1 Remes J, Miettinen H, Reunanen A, Pyorala K. Validity of 13 Coughlin SS, Labenberg JR, Tefft MC. Black-white the clinical diagnosis ofheart failure in primary health care. differences in IDCM. Epidemiology 1993; 4: 165-72. Eur HeartJ1 1991; 12: 315-21. 14 Figulla HR, Kellerman AB, Stille-Siegener M, et al. 2 Kelly TL, Cremo R, Nielson C, Shabetai R. Prediction of Significance of coronary angiograph, left heart catheteriza- outcome in late-stage cardiomyopathy. Am Heart J 1990; tion and for the diagnosis of IDCM. 119: 1111-21. Am Heart a 1992; 124: 1251-7. 3 Codd MB, Sugrue DD, Gersh BJ, Melton LJ III. 15 Banka VS, Bodenheimer MM, Helfant RH. Determinants of Epidemiology of idiopathic dilated and hypertrophic car- reversible asynergy - the native coronary circulation. Cir- diomyopathy. A population based survey in Minnesota, culation 1975; 52: 810-6. 1975-1984. Circulation 1989; 80: 564-72. 16 Dilzian V, Rocco TP, Freedman NM, Leon MB, Bonow 4 Chakko S, Gheorghiade M. Estimating the severity of RO. Enhanced detection of ischaemic but viable myocar- chronic heart failure: a clinical challenge for the 1990s. Am dium by the reinjection of thallium after stress- Heart J 1992; 124: 260-4. redistributing imaging. N Engl J Med 1990; 323: 141-6. 5 Elefteriades JA, Tolis G, Levi E, Mills LK, Zaret BL. 17 Iskandrian AS, Hakki A, Kane SA, et al. Rest and redist- Coronary artery bypass grafting in severe left ventricular ribution thallium myocardial scintigraphy to predict im- dysfunction. J Am Coll Cardiol 1993; 22: 1411-7. provement in left ventricular function after coronary artery http://pmj.bmj.com/ 6 Piggott JD, Kouchoukos NT, Oberman A, Cutter GR. Late bypass grafting. Am J Cardiol 1983; 51: 1312-6. results of surgical and medical therapy in patients with 18 Tillisch JH, Brunken R, Marshall R, et al. Reversibility of coronary artery disease and depressed left ventricular func- cardiac wall motion abnormalities predicted by PET. NEngl tion. J Am Coll Cardiol 1985; 5: 1036-45. J Med 1986; 314: 884-8. 7 Moraes CT, DiMauro S, Zeviani M et al. Mitochondrial 19 Bonow RO, Dilsizian V, Cuocolo A, Bacharach SL. DNA deletions in progressive external ophthalmoplegia and Identification of viable myocardium in patients with cor- Keams-Sayre syndrome. N EnglI Med 1989; 320: 1293-9. onary artery disease and left ventricular dysfunction: com- 8 Tamburro P, Wilber D. Sudden death in idiopathic dilated parison of thallium and PET imaging. Circulation 1991; 83: cardiomyopathy. Am Heart J 1992; 124: 1035-45. 26-37. 9 William Dec G, Fuster V. Idiopathic dilated car- 20 Tamaki N, Ohtani H, Yamashita K, et al. Metabolic activity diomyopathy. N Engl J Med 1994; 331: 1564-75. in the areas of new fill-in after thallium-201 reinjection: on September 30, 2021 by guest. Protected copyright. 10 Benton RE, Coughlin SS, Tefft MC. Predictors of coronary comparison with PET using fluorine 18-deoxyglucose. J7 angiography in patients with IDCM: the Washington DC Nucl Med 1991; 32: 673-8. dilated cardiomyopathy study. J Clin Epidemiol 1994; 47: 21 Dreyfus G, Duboc A, Blasco C, et al. Coronary surgery can 501-11. be an altemative to in selected patients 11 Hannan EL, Kilburn H, O'Donnell JF, et al. Interracial with end-stage ischaemic heart disease. Eur J Cardiothorac access to selected cardiac procedures for patients hos- Surg 1993, 7: 482-8. pitalized with coronary artery disease in New York State. 22 Carmelo A, Milano MD, William D, et al. Coronary artery Med Care 1991; 29: 430-41. bypass in patients wtih severely depressed ventricular func- 12 Ford E, Cooper R, Castaner A, et al. Coronary arteriography tion. Ann Thorac Surg 1993; 56: 487-93. and coronary bypass surgery among whites and other racial 23 Magovern JA, Magovern GJ, Maher TD, et al. Operation groups relative to hospital-based rates for CAD: findings for congestive heart failure: transplantation, bypass, and from NHDS. Am J Public Health 1989; 79: 437-40. cardiomyoplasty. Ann Thorac Surg 1993; 56: 418-25.